Korean J Lab Med.  2006 Apr;26(2):119-122. 10.3343/kjlm.2006.26.2.119.

A Splenectomy in Plasma Exchange Refractory Thrombotic Thrombocytopenia Purpura

• Lee YJ
• Shim H
• Park DE

• KMID: 2238902
• DOI: http://doi.org/10.3343/kjlm.2006.26.2.119

Abstract

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, and variable abnormalities in renal function and mental status. The pathogenesis of TTP is related to an inhibitor or deficiency of the von Willebrand factor (vWF)-cleaving protease (a disintegrin and metalloprotease with thrombospondin type 1 repeats; ADAMTS-13) that cleaves the large vWF multimers. Uncleaved, large vWF molecules are present in TTP and induce thrombosis in small vessels. Even though plasma exchange was proven effective in TTP, 20-40% of the cases showed refractory to plasma exchange. We describe a 41 years old female with plasma exchange refractory TTP who was completely recovered from anemia, thrombocytopenia, and accompanying symptoms following splenectomy.

Keyword

TTP; vWF; ADAMTS-13; Plasma Exchange; Splenectomy

MeSH Terms

Adult
Anemia
Anemia, Hemolytic
Female
Fever
Humans
Plasma Exchange*
Plasma*
Purpura*
Purpura, Thrombotic Thrombocytopenic
Splenectomy*
Thrombocytopenia*
Thrombosis
Thrombospondins
von Willebrand Factor
Thrombospondins
von Willebrand Factor

Figure

• 

  Fig. 1. The sequential change of the value of lactate dehydrogenase (▴) and platelet number (Δ) before and after plasma exchanges, splenectomy (), vincristine (), and steroid therapy.

Reference

References

1. Moschowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc New York Pathol Soc. 1924; 24:21–4.

2. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997; 89:3097–103.

3. Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, et al. Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med. 1982; 307:1432–5.

4. Furlan M, Robles R, Solenthaler M, Lammle B. Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood. 1998; 91:2839–46.

5. Yoon SY, Hahn JS, Lee SK, Lee S, Chong SY, Yoo NC, et al. A case of splenectomy in a patient with chronic relapsing thrombotic thrombocytopenic purpura. Korean J Hemost Thromb. 1997; 4:79–84.

6. Choi YB, Min SJ, Ahn SM. Should open splenectomies still only be recommended in benign hematologic diseases refractory to medical therapy? J Korean Surg Soc. 2004; 66:231–8.

7. Aqui NA, Stein SH, Konkle BA, Abrams CS, Strobl FJ. Role of splenectomy in patients with refractory or relapsed thrombotic thrombocytopenic purpura. J Clin Apheresis. 2003; 18:51–4.

8. Hovinga JA, Studt JD, Biasiutti FD, Solenthaler M, Alberio L, Zwicky C, et al. Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura. Haematologica. 2004; 89:320–4.

9. Tsai HM, Lian EC. Antibodies to von Willebrand factor cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998; 339:1585–94.

10. Fujiwaka K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001; 98:1662–6.

11. Sadler JE. A new name in thrombosis: ADAMTS13. Proc Natl Acad Sci. 2002; 99:11552–4.

12. Furlan M, Robles R, Lamie B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996; 87:4223–34.

13. Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001; 413:488–94.

14. Mannucci PM, Canciani MT, Forza I, Lussana F, Lattuada A, Rossi E. Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood. 2001; 98:2730–5.

15. Hovinga JA, Studt JD, Lammle B. The von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP). Pathophysiol Haemost Thromb. 2003; /2004;. 33:417–21.

16. Pimanda JE, Chesterman CN, Hogg PJ. A perspective on the measurement of ADAMTS13 in thrombotic thrombocytopaenic purpura. Eur J Haematol. 2003; 70:257–62.